Glomeruloid haemangioma and POEMS syndrome.

A 49-year-old milkman presented after the acute onset of blurred vision, anorexia, malaise, weight loss and ankle swelling. There was also a 4-month history of progressive dyspnoea, weakness, parasthesiae, tinnitus and hearing loss and earlier nerve conduction studies had revealed a demyelinating peripheral neuropathy. After a normal CT scan, undertaken because the patient had papilloedema, the patient’s blood pressure (200u120) was treated with atenolol and he was referred to the Centre for Nephrology at the Royal Free and University College Medical School. On examination he had a 6 mm purple nodule on the anterior chest wall and gynaecomastia. Blood pressure was 130u95, the patient was b-blocked and loud P2 and S3 heart sounds were heard. Ankle and sacral oedema, bilateral pleural effusions and ascites were present but there was no palpable organomegaly or lymphadenopathy. Fundoscopy showed bilateral papilloedema and a single right flame-shaped haemorrhage. The blind spot was enlarged bilaterally and Rinne’s test was positive bilaterally. Examination of the peripheral nervous system revealed upper limb hyporeflexia and proximal loss of power, absent reflexes, and reduced distal sensation in the legs. Routine investigations demonstrated significant renal impairment, urea 21 mmolul (0.5–4.7), creatinine 174 mmolul (60–120) without significant proteinuria (0.14 g in 24 h), a neutrophilia, a raised alkaline phosphatase of 199 (50–130 Uul) and an acute inflammatory response with ESR 50 mmuh (0–15), CRP 60 mgul (0–5). Haemoglobin, platelets, albumin, bilirubin, transaminases, clotting studies, calcium, a myeloma screen and creatinine kinase were normal. Echocardiography confirmed the clinical diagnosis of pulmonary hypertension. CSF protein was raised (2.6 gul), and matching IgG oligoclonal bands were found in CSF and serum. Hypogonadotrophic hypogonadism and primary hypothyroidism were found during the investigation of the patient’s gynaecomastia. Ultrasound demonstrated splenomegaly and normal-size kidneys. Light microscopic examination of the renal biopsy (Figure 1) showed glomerular hypercellularity, with endocapillary and mesangial proliferation, accentuation of the lobular outline of the glomerular tuft and mild focal interstitial fibrosis. Silver staining revealed capillary loop irregularity but double contours and spikes were not present. Immunofluorescence showed scanty mesangial granules of C3. No immunoglobulin deposition was identified. Electron microscopy showed mesangial expansion and interposition of mesangial cells between endothelium and new basement membrane. No electron dense deposits were seen. There was a marked electron-lucent expansion of the sub-endothelial space of the glomerular capillaries (Figure 2). The diagnosis in this case was suggested by biopsy of the lesion found on the patient’s chest. Review of the biopsy in the histopathology and dermatology departments suggested that the appearances were consistent with a glomeruloid haemangioma, a lesion strongly associated with POEMS syndrome (Figures 3 and 4). The patient was immunosuppressed with prednisolone and azathioprine, and hypertension treated with nifedipine and bendrofluazide. He was anticoagulated and replacement treatment with thyroxine and testosterone patches initiated. This regimen has been successful and the patient is left with minimal residual